Epidemiology

RCC is the 7th leading cancer in men and 12th leading cancer in women. About 2% are associated with inherited conditions and the other 98% are spontaneous.

Pathophysiology

RCC arises from the renal epithelium and accounts for 85% of renal cancers.

Risk Factors

• Smoking
• Obesity
• Hypertension
• Acquired cystic kidney disease with ESRD
• Male sex
• Age 60-70s

Diagnosis

Clinical Manifestations

• classic triad of flank pain, hematuria, and a palpable abdominal mass
• often asymptomatic and radiographically detected incidentally
  • CT KUB in all people < 40 with any hematuria + Cystoscopy in people > 40 to rule out bladder cancer

Imaging

• enhancing renal mass on a CT scan obtained after the administration of contrast material is a strong clue that renal cancer is present
• staging workup
• hereditary predisposition suggested by multiple lesions, family history of RCC

Screening and Followup

• consider the VHL disease, hereditary leiomyomatosis and renal cell cancer, and Birt-Hogg-Dube syndromes
  • uterine tumours
  • renal cysts, pancreatic, adrenal lesions (VHL)
• VHL disease --> MR of the brain and spinal cord to screen for hemangioblastoma
• immediately excise tumours from hereditary leiomyomatosis and RCC syndrome (aggressve)
• otherwise follow tumours from hereditary RCC using serial imaging until >3cm at which time they should be removed
• family pedigree should be generated

Classification

The Heidelberg classification correlates histopathological features with genetic defects.

Clear-Cell RCC

• VHL disease (retinal angiomas, hemangioblastomas of the CNS, pheochromocytomas, and RCC) is classically associated with RCC
• Spontaneous mutations in VHL are responsible for 60% of spontaneous clear-cell RCC.

Papillary RCC

• sporadic papillary RCC has a 5-year survival rate 90% and 5:1 male predominance
• Localized papillary RCC metastasizes less frequently than clear-cell RCC. However, the survival rate for metastatic papillary RCC is probably worse than that for clear-cell RCC
• the MET proto-oncogene is implicated in 75% of sporadic cases

Prognosis

1/4 present with advanced disease (locally invasive or metastatic), and recurrence rates are high post resection of localized disease. Median survival for patients with metastatic disease is ~13 months.

Treatment

Surgical Approaches

• radical nephrectomy +/- surgical excision of solitary met
• nephron-sparing partial nephrectomy
• percutaneous ablative procedures (RF heat ablation or cryoablation)

Medical Treatment

• offered for locally advanced or metastatic RCC
• 

References

Cohen and McGovern, NEJM 2005